An annotated and illustrated check-list of species of the subfamily Sphaeriinae (family Sphaeriidae) of the Kama region (the Middle Urals, Russia), with enumeration of species’ localities, is presented. After examination of own collections and critical assessment of literary data, 8 species from two genera (Musculium and Sphaerium) have been included into the final list. One of these species, Sphaerium asiaticum, is recorded for the first time from the studied area. The ecological characteristics of all species as well as remarks on their distribution within the region are provided. From the zoogeographical point of view, the most sphaeriine species of the studied area belong to the European-Siberian faunistic group.